Abstract
Sézary syndrome is an aggressive leukemic variant of cutaneous T-cell lymphomas, characterized by erythroderma, lymphadenopathy, and peripheral blood involvement by CD4+ malignant T-cells. The pathogenesis of Sézary syndrome is not fully understood. However, the course of the disease is strongly influenced by the tumor microenvironment, which is altered by a combination of cytokines, chemokines, and growth factors. The crosstalk between malignant and reactive cells affects the immunologic response against tumor cells causing immune dysregulation. This review focuses on the interaction of malignant Sézary cells and the tumor microenvironment.
Highlights
Sézary syndrome (SS) was first described by Albert Sézary and Yves Bouvrain in1938 [1]
The Treg properties expressed by Sézary cells are stimulated by staphylococcal enterotoxins (SEs), which trigger the expression of FoxP3 in a STAT5-dependent manner, and by direct contact with immature DCs via major histocompatibility complex (MHC) class 2 presentation of antigens of apoptotic cells
Different genetic and epigenetic alterations in SS are described and, despite the heterogeneous findings, they converge to the JAK/STAT pathway alterations, with an increased STAT3, STAT5, and STAT6 and decreased STAT4 activation
Summary
MF cells strongly express C-C chemokine receptor (CCR)-4 and cutaneous lymphocyte-associated antigen (CLA), which confer tropism to the skin, and are negative for CCR7 and L-selectin, receptors that confer tropism to the lymph nodes. This immunophenotype is characteristic of skinresident memory T-cells. Sézary cells express CCR7 and L-selectin, CD27 (a characteristic marker of central memory T-cells), CCR4, and other skin-tropic receptors (CCR6, CCR10, CLA) These findings suggest that MF and SS originate from different subtypes of T lymphocytes [10]. This review focuses on the discussion of the current scenario of the immunologic milieu of SS
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